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Extranodal natural killer/T-cell lymphoma (ENKTL) is an aggressive neoplasm derived from cytotoxic T cells or natural killer (NK) cells, with higher prevalence in Asia and Latin America. Epstein-Barr virus (EBV) infection plays a crucial role in its pathogenesis, being detected in most cases through in situ hybridization for EBV-encoded RNA (EBER). ENKTL predominantly affects men between 40 and 50 years of age, mainly involving the upper aerodigestive tract, particularly the nose and nasopharynx. However, it can also present in extranasal sites, where it tends to behave more aggressively. Clinical manifestations include cutaneous lesions with variable presentations that often mimic infections, delaying diagnosis. In limited-stage disease, radiotherapy (RT) is the treatment of choice, while advanced disease requires chemotherapy regimens, although no universally accepted standard protocol currently exists.